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Treatment of optic pathway hypothalamic gliomas in childhood: experience with 18 consecutive cases
Tang-Her Jaing1, Kuang-Lin Lin, Pei-Kwei Tsay
1Division of Hematology and Oncology, Department of Pediatrics, Chang Gung Children's , Chang Gung University, Taoyuan, Taiwan. jaing001@cgmh.org.tw
Insights
Optic pathway/hypothalamic gliomas (OPHG) in children often show slow progression and can lead to endocrine issues. Chemotherapy is recommended as a primary treatment for progressing OPHG due to sustained tumor shrinkage and survival benefits.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Ophthalmology
Background:
- Optic pathway/hypothalamic gliomas (OPHG) are rare pediatric tumors.
- Long-term data on treatment outcomes and sequelae are crucial for managing these complex cases.
Purpose of the Study:
- To review a 17-year experience in treating pediatric OPHG.
- To evaluate treatment modalities, outcomes, and long-term sequelae.
Main Methods:
- Retrospective analysis of 18 pediatric patients with OPHG treated between 1989 and 2006.
- Diagnosis confirmed by imaging (CT/MRI) and histology (WHO grades I-III).
- Treatment included surgery, chemotherapy, and radiotherapy.
Main Results:
- Low-grade astrocytoma was the most common histology (16/18 cases).
- All treatments achieved tumor shrinkage/stabilization; 78% had sustained reduction.
- 5-year overall survival was 80%, progression-free survival was 63.3%.
- Endocrinologic sequelae (e.g., growth hormone deficiency) affected 56% of patients.
Conclusions:
- OPHG management requires a multidisciplinary approach.
- Chemotherapy is recommended as a primary treatment for progressing OPHG due to its efficacy in tumor control and survival.
- Long-term endocrine monitoring and management are essential for affected children.
Abstract:
The aim of this study was to present our 17-year experience (1989 to 2006) in the treatment of optic pathway/hypothalamic gliomas (OPHG) in 18 children younger than 17 years (median age, 66 mo). Only 2 of these had evidence of neurofibromatosis-1. OPHG was diagnosed using computed tomography and/or magnetic resonance imaging. Histologic studies showed low-grade astrocytoma (WHO grade I or II) in 16 cases, anaplastic astrocytoma in 1, and oligoastrocytoma (WHO grade III) in 1. Treatment included partial tumor resection in 12 patients, chemotherapy in 5, and radiotherapy in 3. Ophthalmologic and visual alterations occurred in 12 patients, endocrine alterations in 6, and neurologic signs in 5. All treatment modalities led to tumor shrinkage and stabilization for a variable period, but none of them totally eradicated the tumor. Fourteen (78%) of 18 patients had a sustained reduction of tumor size between 6 months and 17 years. The 5-year overall and progression-free survival rates were 80.0% and 63.3%, respectively. Fifty-six percent of patients had endocrinologic sequelae, with growth hormone deficiency being the most common. Two patients died, none with neurofibromatosis-1, with a hypothalamic/chiasmatic tumor with suprasellar extension and accompanying electrolyte abnormalities. Because progression of these tumors is slow and associated with endocrinopathy, we recommend chemotherapy as a primary treatment of OPHG if the disease progresses.
